Vick, LL Project Summary/Abstract Hydroxyurea Adherence Project (HAP) Project Summary/Abstract (30 lines or less) Problem. Suboptimal adherence to medications and treatment is associated with increased morbidity and mortality, with associated health care costs estimated between 100 - 300 billion dollars, nationally1. Adherence to hydroxyurea (HU) is a problem for persons with sickle cell disease (SCD). A landmark double-blind randomized, controlled trial demonstrated the benefits of HU use by adult patients with SCD. Participants taking HU experienced a reduction in the frequency of vaso-occlusive crises, hospitalizations, and the need for blood transfusions2. HU is a fetal hemoglobin (HbF) inducer that mitigates many of the chronic complications in persons with SCD and is the principle U.S. FDA approved medication for adults (1998) and children (2017) with SCD.3,4 HU also reduces the episodes of acute chest syndrome, number of hospitalizations, and mortality.5-12 Known. Poor adherence to HU is associated with lower fetal hemoglobin and the protective factors associated with the treatment. Adherence strategies include treatment simplification and decreased dosing (1 or 2 times per day), behavioral interventions given 1 to 1, and linking adherence to routine habits. Proposed Solution. The Hydroxyurea Adherence Project (HAP) is a behavioral pilot intervention study. It can be summarized as a project using a set of behavioral intervention strategies to improve adherence to hydroxyurea (HU) and health outcomes in persons with sickle cell disease. This project is based on the findings from HU adherence literature to determine strategies that may promote HU adherence. The Parent Grant title: Implementation of medical homes for evidence-based care of adolescents and adults with sickle cell disease. The parent grant authors identified substantial study evidence that supports the benefit of HU. Currently, acceptance of HU is inadequate, given the considerable disease burden and early mortality associated nonadherence. Strategies to increase adoption of the SCD clinical practice guidelines13 including HU use within a Patient-Centered Medical Neighborhood model is an important goal. HU adherence can change the course of disease progression in persons with sickle cell disease and thus result in a healthier life. The goal of this project is to improve adherence to HU therapy by empowering the person with SCD in behavior change that supports adherence to hydroxyurea for the person?s health benefit. Vick, LL Project Summary/Abstract Hydroxyurea Adherence Project (HAP) References 1. Iuga AO, McGuire MJ. Adherence and health care costs. Risk Manag Healthc Policy. 2014; 7:35?44. Published 2014 Feb 20. doi:10.2147/RMHP.S1980 2. Charache S, Barton FB, Moore RD, Terrin ML, Steinberg MH, Dover GJ, Ballas SK, McMahon RP, Castro O, Orringer EP. Hydroxyurea and sickle cell anemia. Clinical utility of a myelosuppressive switching agent. The Multicenter Study of Hydroxyurea in Sickle Cell Anemia. Medicine. 1996 Nov;75(6):300-26. 3. U.S. Food and Drug Administration. Drugs@FDA: FDA approved drug products. 1998. https://www.accessdata.fda.gov/scripts/cder/daf/index.cfm?event=overview.process&applno =075143#collapseTE 4. U.S. Food and Drug Administration. Approved drugs: FDA approves hydroxyurea for treatment of pediatric patients with sickle cell anemia. 2017 https://www.fda.gov/Drugs/InformationOnDrugs/ApprovedDrugs/ucm590096.htm 5. Al-Anazi KA. Hydroxyurea therapy in patients with sickle cell disease. Transl Med (Sunnyvale). 2015; 5:145-50. 6. Anders DG, Tang F, Ledneva T, Caggana M, Green NS, Wang Y, Sturman LS. Hydroxyurea use in young children with sickle cell anemia in New York State. Am J Prev Med. 2016; 51(1): S31-8. https://doi.org/10.1016/j.amepre.2016.01.001 7. Ballas, SK. Sickle Cell Pain. 2nd Ed. Washington DC: International Society for the Association of Pain (IASP) Press. 2014. 8. Creary, S E, Gladwin, M T, Byrne, M, Hildesheim, M, Krishnamurti, L. A pilot study of electronic directly observed therapy to improve hydroxyurea adherence in pediatric patients with sickle-cell disease: Electronic DOT for Hydroxyurea. Pediatr Blood Cancer. 2014:61(6), 1068?1073. doi.org/10.1002/pbc.24931. 9. Estepp JH, Winter B, Johnson M, Smeltzer MP, Howard SC, Hankins JS. Improved hydroxyurea effect with the use of text messaging in children with sickle cell anemia. Pediatr Blood Cancer. 2014;61(11):2031?2036. doi.org/10.1002/pbc.25177. 10. Green NS, Manwani D, Matos S, et al. Randomized feasibility trial to improve hydroxyurea adherence in youth ages 10?18 years through community health workers: The HABIT study. Pediatr Blood Cancer. 2017;64: e26689. doi.org/10.1002/pbc.26689. 11. Inoue S, Kodjebacheva G, Scherrer T, et al. Adherence to hydroxyurea medication by children with sickle cell disease (SCD) using an electronic device: A feasibility study. Int J Hematol. 2016;104(2):200?207. doi.org/10.1007/s12185-016-2027-x. 12. Olivieri, NF & Vichinsky, EP Hydroxyurea in children with sickle cell disease: impact on splenic function and compliance with therapy. J Pediatr Hematol Oncol. 1998;20(1):26?31. 13. U.S. Department of Health and Human Services, National Institutes of Health, National Heart, Lung, and Blood Institute. Evidence-based management of sickle cell disease: expert panel report. http://www.nhlbi.nih.gov/health-pro/guidelines/sickle-cell-disease-guidelines. 2014.